Corpus callosotomy for treatment of pediatric refractory status epilepticus.

Medically refractory status epilepticus (RSE) causes high morbidity and mortality in children. There are no evidence-based guidelines for treatment. Epilepsy surgery is a treatment option for RSE. We describe a 9-year-old boy treated successfully for RSE with complete corpus callosotomy (CC). Epilepsy surgery should be considered for prolonged RSE. In the absence of evidence of focal epileptogenesis, complete corpus callosotomy may be effective in select cases.

Use of a mild traumatic brain injury guideline to reduce inpatient hospital imaging and charges.

PURPOSE: In children, mild traumatic brain injuries (TBI) account for 70% to 90% of head injuries. Without clear guidelines, many of these children may be exposed to excess radiation owing to unnecessary imaging. The purpose of this study was to evaluate the impact of a mild TBI guideline in reducing hospital charges and repeated imaging of pediatric patients. METHODS: Charts of all children who had at least one head computed tomography and were admitted to our level 1 trauma center with a blunt TBI and Glasgow Coma Scale of 13 to 15 were retrospectively reviewed. Patients were divided into 2 groups relative to the implementation of a TBI management guideline. RESULTS: A total of 742 patients were included, 389 preguideline and 353 postguideline. Implementation of the guideline was associated with reductions in the average number of head computed tomographies performed (1.6 vs 1.3, P = .006), length of stay (2.3 vs 1.7 days, P < .0001), and overall hospital charges ($21,760 vs $13,980, P = .006). No children were readmitted for missed injuries. CONCLUSIONS: Implementation of a simple guideline for the care of children with mild TBI can have significant impact on charges and length of stay while simultaneously reducing radiation exposure. Widespread implementation of such guidelines will improve efficiency without sacrificing quality of care in the management of mild TBI in the pediatric population.

Medulloblastoma with melanotic differentiation: case report and review of the literature.

Medulloblastoma with melanotic differentiation, a rare variant of medulloblastoma, often carries a poor prognosis. We present such a case of a 4 year male with this rare, aggressive tumor. Additionally, we have reviewed the literature and report on the features important in the pathologic and radiologic diagnosis in this type of tumor, as well as review clinical outcomes. This subtype of medulloblastoma occurs more frequently in males, at a younger median age than the other subtypes of medulloblastoma. The prognosis is generally very poor. However, it is important to note, that a subset of patients with M0 disease who can achieve a gross total resection followed by radiation and platinum based chemotherapy can become long term survivors of this aggressive subtype of medulloblastoma.

Cystic degeneration of the cerebellar tonsils in pediatric patients with Chiari Type I malformation.

OBJECT: The operative indications and treatment algorithms for pediatric patients with Chiari Type I malformation (CM-I) vary widely. When an intradural approach and duraplasty are thought necessary at the time of surgery, neurosurgeons may elect to fulgurate or resect a portion of the cerebellar tonsils. Histological analyses of cerebellar tonsils resected during decompression in pediatric patients with CM-I have revealed multiple abnormal findings including extensive ischemic and degenerative changes. The authors describe an interesting phenomenon of cystic degeneration in the distal ends of the cerebellar tonsils in children undergoing operative treatment of CM-I. METHODS: The authors reviewed the clinical database of 440 pediatric patients who underwent surgical decompression for CM-I performed by a single surgeon. The clinical course, preoperative MR imaging and intraoperative ultrasound characteristics, and histological findings in 3 children found to have tonsillar cystic degeneration were analyzed and detailed. RESULTS: Cystic changes were subtle but uniformly evident on preoperative MR imaging and were more readily apparent on intraoperative ultrasonography. In each patient, the tonsillar cyst was resected. Histological examination revealed areas of cystic degenerative change characterized by distortion of the normal cerebellar architecture with absent Purkinje and internal granular cell layers. All children experienced improvement in their symptoms, without complication, postoperatively. CONCLUSIONS: Cystic degeneration of the tonsils in pediatric patients with CM-I is an uncommon pathological process most likely resulting from long-standing and excessive compression. Based on their experience, the authors advocate expeditious surgical treatment, including intradural exploration and capacious duraplasty, for patients in whom there is evidence of this phenomenon on preoperative imaging.

Posterior vertebral column subtraction osteotomy: a novel surgical approach for the treatment of multiple recurrences of tethered cord syndrome.

Recurrent tethered cord syndrome (TCS) has been reported to develop in 5-50% of patients following initial spinal cord detethering operations. Surgery for multiple recurrences of TCS can be difficult and is associated with significant complications. Using a cadaveric tethered spinal cord model, Grande and colleagues demonstrated that shortening of the vertebral column by performing a 15-25-mm thoracolumbar osteotomy significantly reduced spinal cord, lumbosacral nerve root, and terminal filum tension. Based on this cadaveric study, spinal column shortening by a thoracolumbar subtraction osteotomy may be a viable alternative treatment to traditional surgical detethering for multiple recurrences of TCS. In this article, the authors describe the use of posterior vertebral column subtraction osteotomy (PVCSO) for the treatment of 2 patients with multiple recurrences of TCS. Vertebral column resection osteotomy has been widely used in the surgical correction of fixed spinal deformity. The PVCSO is a novel surgical treatment for multiple recurrences of TCS. In such cases, PVCSO may allow surgeons to avoid neural injury by obviating the need for dissection through previously operated sites and may reduce complications related to CSF leakage. The novel use of PVCSO for recurrent TCS is discussed in this report, including surgical considerations and techniques in performing PVCSO.

Management of vagal nerve stimulator infections: do they need to be removed?

OBJECT: Vagal nerve stimulators (VNSs) have been used successfully to treat medically refractory epilepsy. Although their efficacy is well established, appropriate management of infections is less clearly defined. In the authors' experience, patients who have gained a benefit from VNS implantation have been reluctant to have the device removed. The authors therefore sought conservative management options to salvage infected VNS systems. METHODS: The authors performed a retrospective review of 191 (93 female and 98 male) consecutive patients in whom VNS systems were placed between 2000 and 2007. RESULTS: They identified 10 infections (5.2%). In 9 of 10 patients the cultured organism was Staphylococcus aureus. Three (30%) of 10 patients underwent early removal (within 1 month) of the VNS as the initial treatment. The remaining 7 patients were initially treated with antibiotics. Two (28.6%) of these patients were successfully treated using antibiotics without VNS removal. Patients in whom conservative treatment failed were given cephalexin as first-line antibiotic treatment. All patients recovered completely regardless of treatment regimen. CONCLUSIONS: This study confirms the low rate of infection associated with VNS placement and suggests that, in the case of infection, treatment without removal is a viable option. However, the authors' data suggest that oral antibiotics are not the best first-line therapy.

Characterization of abnormal diffusion properties of supratentorial brain tumors: a preliminary diffusion tensor imaging study.

OBJECT: Diffusion tensor (DT) imaging was used in children with supratentorial tumors to evaluate the anisotropic diffusion properties between different tumor grades and between tumors and adjacent and contralateral white matter. METHODS: In this retrospective review, the authors review the cases of 16 children (age range 1-18 years) who presented to their institution with supratentorial tumors and were treated between 2004 and 2007. Eleven patients had low-grade and 5 had high-grade tumors. Fractional anisotropy (FA), mean diffusivity, and axial (lambda parallel) and radial (lambda perpendicular) eigenvalues within selected regions were studied. Mitotic index, necrosis, and vascularity of the tumors were compared with DT imaging parameters. RESULTS: The mean diffusivity was significantly higher in low-grade than in high-grade tumors (p = 0.04); the 2 tumor grades also significantly differed for both lambda parallel (p < 0.05) and lambda perpendicular (p < 0.05). Mean diffusivity values in low-grade tumors were significantly higher than in adjacent normal-appearing white matter (NAWM; p = 0.0004) and contralateral NAWM (p = 0.0001). In both low- and high-grade tumors, the FA was significantly lower than in NAWM (p < 0.0001 and p < 0.03, respectively) and contralateral NAWM (p < 0.0001 and p < 0.003, respectively). Tumor cellularity highly correlated with mean diffusivity and lambda parallel and lambda perpendicular. CONCLUSIONS: Diffusion tensor imaging is a useful tool in the evaluation of supratentorial tumors in children. The mean diffusivity appears to be a significant marker in differentiating tumors grades. Findings related to lambda parallel and lambda perpendicular within tumor groups and between tumors and NAWM may be an indirect manifestation of the combined effects of axonal injury, demyelination, and tumor mass within the cranial compartment.

Spontaneous extracranial decompression of epidural hematoma.

Epidural hematoma (EDH) is a common sequela of head trauma in children. An increasing number are managed nonsurgically, with close clinical and imaging observation. We report the case of a traumatic EDH that spontaneously decompressed into the subgaleal space, demonstrated on serial CT scans that showed resolution of the EDH and concurrent enlargement of the subgaleal hematoma.

Postlaminectomy kyphosis in an achondroplastic adolescent treated for spinal stenosis.

We report a case of a skeletally immature achondroplastic adolescent with significant thoracolumbar lordosis who presented with neurogenic claudication and urinary incontinence progressing over a 1-year period. She underwent decompressive lumbar laminectomy from T12 to L5 with preservation of the facet joints. Over the ensuing 4 years of follow-up, she developed a progressive thoracolumbar kyphosis that progressed to 105 degrees. She remained neurologically intact and was fully ambulatory, but had severe back pain in the area of the deformity. Because of the severity and progression of her deformity, a combined anterior and posterior fusion and stabilization was required. We present this case and a review of the literature on spinal stenosis in achondroplasia and the complication of post-laminectomy kyphosis.

Intraoperative ultrasonography used to determine the extent of surgery necessary during posterior fossa decompression in children with Chiari malformation type I.

OBJECT: In this retrospective analysis, the authors report a prospective study in which intraoperative ultrasonography was used to determine the extent of surgery necessary during posterior fossa decompression surgery for Chiari malformation Type I (CM-I) in children. METHODS: Between 1995 and 2003, posterior fossa decompression was performed in 149 patients (mean 5.9 years of age, range 9 months-18 years of age) with CM-I. Of these, 130 underwent intraoperative ultrasonographic evaluation of the craniocervical junction (CCJ) and 15 did not. Four patients with craniosynostosis were excluded from the study. Duraplasty and tonsillar shrinkage were performed when ultrasonographic evidence showed significant decreases in cerebrospinal fluid (CSF) or abnormal tonsillar piston action. Surgical success was determined on the basis of clinical outcome and need for reoperation. One hundred and twenty-four (95.5%) of the children had successful outcomes following surgery and six (4.5%) experienced continued or worsening symptoms requiring reoperation. Forty patients did not undergo duraplasty because the ultrasonography evidence showed adequate decompression with bone removal alone. Of 90 patients with significant compression, decreased CSF dynamics, and/or abnormal tonsillar piston-like action at the CCJ, 85 underwent duraplasty and tonsillar shrinkage and five did not for various reasons. One patient in whom the dura mater was violated accidentally during bone decompression subsequently underwent duraplasty. Hospital stays lasted 6.4 +/- 3.9 days (mean +/- standard deviation) when duraplasty was performed compared with 4.3 +/- 1.1 days when it was not (p < 0.0003). After bone decompression alone, no patient experienced complications. After duraplasty, 12 patients experienced complications and had headaches, nausea, and pain more often than patients who underwent bone decompression alone. Mean tonsillar descent was 11 +/- 4 mm after bone decompression only and 13.9 +/- 4.9 mm after duraplasty, with tonsillar shrinkage (p < 0.0003) seen on magnetic resonance imaging. CONCLUSIONS: In patients who undergo decompressive surgery for CM-I, intraoperative ultrasonography may be a useful tool to aid the surgeon in deciding whether to opt for bone removal only or bone removal plus duraplasty and tonsillar shrinkage.

Rapamycin causes regression of astrocytomas in tuberous sclerosis complex.

OBJECTIVE: Tuberous sclerosis complex (TSC) is a genetic disorder characterized by the formation of hamartomas in multiple organs. Five to 15% of affected individuals display subependymal giant cell astrocytomas, which can lead to substantial neurological and postoperative morbidity due to the production of hydrocephalus, mass effect, and their typical location adjacent to the foramen of Monro. We sought to see whether therapy with oral rapamycin could affect growth or induce regression in astrocytomas associated with TSC. METHODS: Five subjects with clinically definite TSC and either subependymal giant cell astrocytomas (n = 4) or a pilocytic astrocytoma (n = 1) were treated with oral rapamycin at standard immunosuppressive doses (serum levels 5-15 ng/ml) from 2.5 to 20 months. All lesions demonstrated growth on serial neuroimaging studies. Magnetic resonance imaging scans were performed before and at regular intervals following initiation of therapy. RESULTS: All lesions exhibited regression and, in one case, necrosis. Interruption of therapy resulted in regrowth of subependymal giant cell astrocytomas in one patient. Resumption of therapy resulted in further regression. Treatment was well tolerated. INTERPRETATION: Oral rapamycin therapy can induce regression of astrocytomas associated with TSC and may offer an alternative to operative therapy of these lesions.

Rubinstein-Taybi syndrome in children with tethered spinal cord.

OBJECT: The authors identified eight patients with Rubinstein-Taybi syndrome (RTS) and examined how the underlying conditions of this syndrome can impede the detection of symptoms associated with a tethered spinal cord. They also reviewed the literature and did not find any published series describing such an association with this syndrome. METHODS: In this retrospective review, the authors report the diagnosis, treatment, and outcome of tethered spinal cord in eight children (age range 2-14 years) with RTS. The patients presented between 1995 and 2003 with neurogenic bladder, decreased activity, gait disturbance, and low-back pain. The children were examined using whole-spine magnetic resonance (MR) imaging and, in recent years, their spinal cord movement was assessed using cine MR imaging. After undergoing lumbar laminectomy and spinal cord untethering procedures, six patients experienced improvement in symptoms associated with tethered spinal cord such as pain, urinary tract infection, incontinence, spasticity, and neurogenic bladder. CONCLUSIONS: Although some underlying conditions of RTS may mask the symptoms of a tethered spinal cord in children, careful attention to the patient's medical history as provided by his or her parents may aid in early diagnosis, evaluation, and treatment, thus preventing further neurological deterioration and leading to improved outcomes.

Epidemiology of hemimegalencephaly: a case series and review.

Hemimegalencephaly (HME) is a congenital brain malformation characterized by unilateral enlargement of the cerebral hemisphere. Clinically, HME is typically associated with hemiparesis, psychomotor retardation, and intractable seizures usually apparent soon after birth. HME is often an isolated finding, but it has been described as an occasional feature of a large number of syndromes, many of which may not be readily identified at birth. There are a multitude of case series and reports of HME in the English literature; however, there is no comprehensive, unbiased, detailed survey characterizing the proportion of cases of HME that are associated with a syndrome. We performed a retrospective study of all cases of HME seen at our institution from 1990 to 2003. Of the 15 cases of HME identified, 53% (8/15) were non-syndromic and 47% (7/15) of the cases were associated with a known or suspected genetic syndrome. In patients with syndromic HME, many of the syndromic features were not readily discernible at birth or in early infancy. It is, therefore, imperative to continually evaluate any infant with HME for signs and symptoms of these and other syndromes. Knowing the relative differential diagnosis will lead to a more comprehensive evaluation, improvement in expectant management, and appropriate counseling of families before considering radical surgical options such as hemispherectomy.

Minimally invasive management of a traumatic artery aneurysm resulting from shaken baby syndrome.

Based on our review of the literature, we present the first use of coiling in an infant with a traumatic artery aneurysm that resulted from shaken baby syndrome. Computed tomography (CT) scans showed a skull fracture, hemorrhagic subdural collections, multiple parenchymal contusions, and intraventricular and subarachnoid hemorrhages in a 3-week-old infant who presented with lethargy, poor feeding, and seizure. These multiple injuries were consistent with shaken baby syndrome. After closed-head injury medical management, including subdural taps, the baby was discharged home. When increasing seizures and hydrocephalus developed 8 months later, CT angiographic scans showed a pseudoaneurysm of the anterior cerebral artery. We successfully occluded the aneurysm with pushable coils placed via a microcatheter and treated the obstructive hydrocephalus with endoscopic third ventriculostomy. We show that minimally invasive radiological and surgical techniques may be effective in managing the sequelae of trauma in children.

Recurrence of cerebral arteriovenous malformations in children: report of two cases and review of the literature.

BACKGROUND: Angiography is considered the standard to assess the treatment success of cerebral arteriovenous malformations (AVMs). When postoperative angiograms show neither residual nidus nor early draining veins, patients are considered cured, and the risks of hemorrhage are eliminated. However, this notion is challenged by the development of recurrent AVMs in children. In our report, 2 children developed recurrent AVMs after undergoing complete resection, which was documented by postoperative angiography. We review other similar cases reported in the literature to help establish guidelines for postoperative monitoring. METHODS: In this retrospective review, 36 children underwent complete surgical resection of cerebral AVMs that were documented by intra- or postoperative angiography. After a follow-up period ranging from 1 to 17 years, rates of recurrence were assessed. RESULTS: Although angiographic documentation showed complete resection, 2 children developed recurrences 3 and 5 years later. Among these 36 children, the recurrence rate was 5.5% at follow-up (mean 9 years). CONCLUSIONS: Based on our data and review of the literature, intra- or early postoperative angiography is essential to exclude the presence of a residual nidus but does not ensure cure of the AVMs in children. If postoperative angiographic scans are obtained too early, postoperative changes may prevent the detection of a residual nidus. Therefore, we recommend that angiographic scans be obtained intraoperatively or early postoperatively to document complete resection and again at 1 and 5 years after surgical resection.

Neonatal kyphectomy in the patient with myelomeningocele.

STUDY DESIGN: A retrospective cohort study was used to investigate a group of neonates with myelomeningocele who had a kyphectomy performed in conjunction with dural sac closure during the first few days of life. OBJECTIVES: To assess the effectiveness of operative intervention in the neonatal period to correct the kyphotic deformity in the patient with myelomeningocele and to monitor its long-term results. SUMMARY OF BACKGROUND DATA: Orthopedic management originally focused on the immediate treatment of the kyphotic deformity in the infant with myelomeningocele. However, there has been a movement toward postponing surgical treatment of the kyphos until a later age. This study included the longest follow-up of the largest group of neonates that a single surgeon has managed surgically since the treatment of this condition was originally described. METHODS: The radiographic and clinical results for all neonates treated with a kyphectomy at the time of myelomeningocele closure between 1980 and 2000 were analyzed. RESULTS: Neonatal kyphectomy was performed on nine males and two females. The average preoperative kyphotic angle measured 67 degrees. The average initial correction was 77 degrees, and the average loss of correction at follow-up assessment was 55 degrees. There were no serious complications, and wound closure was successful in all patients. One patient required a repeat kyphectomy and posterior spinal fusion at the age of 9 years and 2 months. The average follow-up period was 7 years and 4 months (range 44-174 months). CONCLUSIONS: Kyphectomy performed at the time of dural sac closure in the neonate is a safe procedure with excellent initial correction. Eventual recurrence is expected despite the procedure. However, it occurs in the form of a longer, more rounded deformity that is less technically demanding.

Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up.

BACKGROUND AND PURPOSE: We herein present our experience in diagnosing and treating 13 children with vein of Galen aneurysmal malformations (VGAM), with an emphasis on possible prognostic indicators, endovascular strategies, factors affecting treatment during the neonatal period, and long-term follow-up. With this review, we hope to identify those factors that have the most significant prognostic value in determining long-term outcomes in children with VGAM. METHODS: We retrospectively reviewed the radiology studies, hospital charts, and outpatient clinic chart notes (when applicable) of 13 children evaluated and treated for VGAM at a single tertiary care pediatric hospital. Clinical presentation, diagnostic methods, treatment strategies, and outcome were documented for each child. The present neurologic status and level of function of each patient was determined by review of the outpatient charts and direct contact with the clinicians who were conducting the follow-up. Outcome was graded on a 5-point scale, ranging from 0 (death) to 4 (normal), taking into account only neurologic and developmental characteristics. RESULTS: Eight of 13 patients presented as neonates with congestive heart failure. The other five patients ranged in age from 4 months to 13 years at the time of presentation. The five patients presenting outside of the neonatal period achieved normal or near-normal outcomes. Two of the eight patients presenting during the neonatal period achieved normal or near-normal outcomes, one experienced significant impairment, and the other five died. We were unable to identify significant differences in outcome on the basis of differences in treatment strategies. CONCLUSION: Our experience confirms that children with VGAM presenting during the neonatal period have a generally much worse prognosis than do those presenting later in childhood. Complicating factors in the management and treatment of these children are discussed in light of their impact on outcome.

Children with suspected craniosynostosis: a cost-effectiveness analysis of diagnostic strategies.

OBJECTIVE: Our purpose was to evaluate the clinical and economic impact of three evaluation strategies in children at different risks of craniosynostosis. MATERIALS AND METHODS: A decision-analytic and cost-effectiveness model was constructed to compare three evaluation in strategies in children with suspected synostosis: no imaging, radiography (if abnormal, followed by three-dimensional CT [3D CT]), and 3D CT. Three risk groups were analyzed on the basis of the prevalence (pretest probability) of disease: low (completly healthy children; prevalence, 34/100,000), intermediate (healthy children with head deformity; prevalence, 1/115), and high risk (children with syndromic craniofacial disorders [i.e., Crouzon's syndrome or Apert's syndrome]; prevalence, 9-10/10). Test performance (sensitivity and specificity) of the evaluation strategies was obtained from the literature. Costs (not charge) estimates were obtained from the hospital cost-accounting database and from the Medicaid fee schedule. RESULTS: In the low-risk group, the radiographic and 3D CT strategies resulted in a cost per quality-adjusted life year (QALY) gained of more than $560,000. In the intermediate-risk group, the radiographic strategy resulted in a cost per QALY gained of $54,600. Three-dimensional CT was more effective than the two other strategies but at a higher cost-hence, with a cost per QALY gained of $374,200. In the high-risk group, 3D CT was the most effective strategy with a cost per QALY gained of $33,800. Less experienced radiologists and poor-quality studies increased the evaluation cost per QALY gained for all of the risk groups because of decreased effectiveness. CONCLUSION: Radiologic screening of completely healthy children (low risk) for synostosis is not warranted because of the high cost per QALY gained of the radiographic and 3D CT strategies. In healthy children with head deformity (intermediate risk), the radiographic strategy had a reasonable cost per QALY gained. Three-dimensional CT was more effective but had a high cost per QALY gained. In children with syndromic craniofacial disorders (high risk), 3D CT was the most effective strategy and had a reasonable cost per QALY gained. Selection of children with suspected craniosynostosis based on their risk group and use of the most appropriate evaluation strategy could maximize clinical and economic outcomes for these patients.